Abstract
The muscular dystrophies (MD) represent an array of inherited myogenic disorders involving mutations and dysregulations of a variety of cytoskeleton or nuclear proteins. Many MD patients also develop a commitant cardiomyopathy. Despite the high incidence of end-stage cardiomyopathy in MD patients there is limited data regarding the mode of maladaptive cardiac remodeling that develops in these patients. In this retrospective study, we examined the hypothesis that MD patients with a cardiomyopathy develop a unique mode of cardiac remodeling as compared to patients with other forms of nonischemic cardiomyopathy. We examined the cardiac MRIs of 3 cohorts (N = 35 per cohort): patients with neuromuscular disorders with associated cardiomyopathy; patients with primary idiopathic nonischemic cardiomyopathy (NICM); and healthy patients from the Dallas Heart Study (DHS). The cardiac MRIs in each group were reviewed, assessing objective measurements of left ventricular ejection fraction (LVEF), mass (LVM), and end diastolic volume (LVEDV). LVM and LVEDV were indexed for body surface area. In addition, left ventricular concentricity 0.67 (LV Conc 0.67 = LVM / LVEDV 0.67 ), a surrogate marker for wall thickness known to decrease in advanced nonischemic heart failure, was computed for each patient. Despite a preserved ejection fraction in the neuromuscular cohort compared to the NICM cohort (58% vs. 30 %, p<0.05), both displayed similar reduced LV Conc 0.67 (4.69 ± 1.05 and 5.16 ± 1.47) compared to the DHS cohort (7.27 ± 0.83). However, whereas the nonischemic cohort demonstrated hypertrophy with marked dilation (95.8 vs. 84.6 g/m 2 and 119.4 vs. 54.7 mL/m 2 , DHS comparison, p<0.05), the neuromuscular cohort demonstrated a reduced cardiac mass (65.6 vs. 84.6 g/m 2 , p<0.05) with only moderate dilation (74.5 vs. 54.7 mL/m 2 , p<0.05) compared to the DHS population. In conclusion, our data suggests that MD patients develop a unique mode of cardiac remodeling and highlights that novel signaling pathways are involved in the development of cardiomyopathy in these patients.
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